Evaluation of renal replacement therapy in children and adolescents in the state of Amazonas, Brazil.

OBJECTIVE: To describe the characteristics of stage-5 chronic kidney disease (CKD) children and adolescents undergoing renal replacement therapy (RRT) in Amazonas, Brazil, estimating the frequencies of current and new cases, describing the presence of anemia and bone metabolism disorders. METHODS: Thirty-five patients aged 7 to 19 years-old on hemodialysis (HD) or peritoneal dialysis (PD) were studied between June 2018 and April 2019. The frequencies of current and new cases were estimated based on the 0 to 19 years-old population of Amazonas, in the same period. Data were collected about the underlying cause and diagnosis of CKD, dialysis, and biochemical analysis. RESULTS: The frequencies of current and new cases were 24 and 15 patients per million people of compatible age (pmpca), respectively. The causes of CKD were nephrotic syndrome (22.8%), nephritic syndrome (14.3%), and neurogenic bladder (14.3%); in 48.6%, the cause was unknown/not investigated. Ten patients underwent renal biopsy, seven with segmental and focal glomerulosclerosis. The majority (80%) were on HD, with an average kt/V of 1.4, and in 51.4% the vascular access was the double lumen catheter. Hypocalcemia was found in 82.8% of patients, hyperphosphatemia in 57.2%, vitamin D insufficiency or deficiency in 60%, and altered parathyroid hormone values in 48.6%. Hemoglobin was low in 80%, with absolute/functional iron deficiency in 28.6%. CONCLUSIONS: In children and adolescents of Amazonas, Brazil, we found 24 pmpca with stage-5 CKU currently in RRT and 16.3 pmpca per year of new cases requiring RRT. Most patients were adolescents on HD, half without a causal diagnosis of CKD, with a high frequency of anemia and bone metabolism disorder.

Collaborative Brazilian pediatric renal transplant registry (CoBrazPed-RTx): A report from 2004 to 2018.

The Brazilian collaborative registry for pediatric renal transplantation began in 2004 as a multicenter initiative aimed at analyzing, reporting, and disseminating the results of pediatric renal transplantation in Brazil. Data from all pediatric renal transplants performed from January 2004 to May 2018 at the 13 participating centers were analyzed. A total of 2744 pediatric renal transplants were performed in the thirteen participating centers. The median age at transplantation was 12.2 years, with the majority being male recipients (56%). The main underlying diseases were CAKUT (40.5%) and glomerulopathy (28%). 1981 (72%) of the grafts were from deceased donors (DD). Graft survival at one year (censored by death) was 94% in the live donor group (LD) and 91% in the DD group (log-rank test P < 0.01). The patient's survival at one and 5 years was 97% and 95% for the LD group and 96% and 93% for the DD group (log-rank test P = 0.02). The graft loss rate was 19% (n = 517), more frequently caused by vascular thrombosis (n = 102) and chronic graft nephropathy (n = 90). DD recipients had 1.6 (1.0-2.2) times greater chance of death and 1.5 (1.2-1.8) times greater chance of graft loss compared to LD recipients. The mortality rate was 5.4% (n = 148), mainly due to infection (n = 69) and cardiovascular disease (n = 28). The results of this collaborative pediatric renal transplant record are comparable to other international registries, although we still have a high infection rate as a cause of death.

Prospective cohort analyzing risk factors for chronic kidney disease progression in children / Coorte prospectiva que analisa os fatores de risco para progressão de doença renal crônica (DRC) em crianças

Abstract Objective: To identify risk factors for chronic kidney disease progression in Brazilian children and to evaluate the interactions between factors. Methods: This was a multicenter prospective cohort in São Paulo, involving 209 children with CKD stages 3-4. The study outcome included: (a) death, (b) start of kidney replacement therapy, (c) eGFR decrease >50% during the followup. Thirteen risk factors were tested using univariate regression models, followed by multivariable Cox regression models. The terms of interaction between the variables showing significant association with the outcome were then introduced to the model. Results: After a median follow-up of 2.5 years (IQR = 1.4-3.0), the outcome occurred in 44 cases (21%): 22 started dialysis, 12 had >50% eGFR decrease, seven underwent transplantation, and three died. Advanced CKD stage at onset (HR = 2.16, CI = 1.14-4.09), nephrotic proteinuria (HR = 2.89, CI = 1.49-5.62), age (HR = 1.10, CI = 1.01-1.17), systolic blood pressure Z score (HR = 1.36, CI = 1.08-1.70), and anemia (HR = 2.60, CI = 1.41-4.77) were associated with the outcome. An interaction between anemia and nephrotic proteinuria at V1 (HR = 0.25, CI = 0.06-1.00) was detected. Conclusions: As the first CKD cohort in the southern hemisphere, this study supports the main factors reported in developed countries with regards to CKD progression, affirming the potential role of treatments to slow CKD evolution. The detected interaction suggests that anemia may be more deleterious for CKD progression in patients without proteinuria and should be further studied.

Resumo Objetivo: Identificar os fatores de risco para progressão da DRC em crianças do Brasil e avaliar as interações entre os fatores. Métodos: Coorte prospectiva multicêntrica em São Paulo, envolvendo 209 crianças com DRC em estágios 3-4. O desfecho do estudo incluiu: a) óbito, b) início da terapia de substituição renal, c) redução de > 50% na taxa estimada de filtração glomerular (eGFR) durante o acompanhamento. Foram testados 13 fatores de risco com o modelo de regressão univariada seguido do modelo de regressão multivariado de Cox. Os termos de interação entre as variáveis mostraram associação significativa e foram introduzidos ao modelo. Resultados: Após média de acompanhamento de 2,5 anos (IIQ = 1,4 a 3,0), 44 casos (21%) apresentaram desfecho: 22 iniciaram diálise, 12 apresentaram redução de > 50% na eGFR, sete foram submetidos a transplante e três morreram. Estágio avançado de DRC no acometimento (RR = 2,16, IC = 1,14-4,09), proteinúria nefrótica (RR = 2,89, IC = 1,49-5,62), idade (RR - 1,10, IC = 1,01-1,17), escore Z da pressão arterial sistólica (RR = 1,36, IC = 1,08-1,70) e anemia (RR = 2,60, IC - 1,41-4,77) foram associados ao resultado. Foi detectada interação entre anemia e proteinúria nefrótica na primeira visita (V1) (RR = 0,25, IC = 0,06-1,00). Conclusões: Como a primeira coorte de DRC no hemisfério sul, este estudo é concordante com os principais fatores relatados em países desenvolvidos com relação à progressão da DRC, afirmando o possível papel dos tratamentos para mostrar a evolução da DRC. A interação detectada sugere que a anemia pode ser mais nociva na progressão da DRC em pacientes sem proteinúria e deve ser ainda mais estudada.

Oral administration of powdered dried rhizomes of Curcuma longa L. (turmeric, Zingiberaceae) is effective in the treatment of doxorubicin-induced kidney injury in rats.

Curcumin is a polyphenol present in the rhizomes of the species Curcuma longa L. ("turmeric," Zingiberaceae), which has been used for centuries as an anti-inflammatory. We aimed to evaluate the anti-inflammatory effects of C. longa in renal injury induced by doxorubicin (DOX, 3.5 mg.kg-1 IV). We studied four groups of Wistar rats: two groups with DOX-induced kidney injury, one fed with standard food and another with standard food mixed with C. longa (5 mg.g-1 ). Two other control groups without kidney injury were fed with the same foods. We measured albuminuria, body weight, and food intake every 2 weeks. After 8 weeks, treatment with C. longa did not change albuminuria, but it significantly attenuated the excretion of urinary inflammatory markers monocyte chemoattractant protein-1 (MCP-1) and transforming growth factor-ß (TGF-ß) and significantly attenuated immunostaining for desmin, vimentin, and ED-1+ cells in renal tissues of rats with DOX-induced kidney injury. In addition, treatment with C. longa resulted in significantly lower glomerular and tubule interstitial injury scores, compared with that in the DOX-STD group. In conclusion, administration of powdered rhizomes of C. longa for 8 weeks to rats with DOX-induced kidney injury did not reduce albuminuria but led to a significant decrease in urinary inflammatory markers MCP-1 and TGF-ß and decreased histopathological alterations and immunostaining for desmin, vimentin, and ED-1+ cells kidneys tissues.

Applicability of Laparoscopic Nephrectomy in the Treatment of Multicystic Dysplastic Kidney: Sorting Out Surgical Indication.

Introduction We evaluated the applicability of laparoscopic nephrectomy in the treatment of multicystic dysplastic kidney (MCDK) in children, including procedures performed by resident physicians or trainees in surgical urology. Methods We retrospectively evaluated the medical records of 20 children with MCDK who underwent laparoscopic nephrectomy over a six-year period. Data collected included gender, laterality of the affected kidney, age at the time of surgery, the largest diameter of the multicystic kidney and associated urological diagnoses, surgical, and postoperative data. All surgical procedures were performed transperitoneally. Results The mean age at the time of surgery was 46 months with a slight predominance of girls. The right side was the more affected side, and the average diameter of the affected kidney at the time of surgical excision was 5.8 cm. Other changes in the urinary tract were found in five children. The mean operative time was 133 minutes. On pathological examination, on average, the pieces measured 4.8 cm and weighed 18.1 g. There were no operative complications. The average length of hospital stay was 37.35 hours. Conclusion Laparoscopic nephrectomy was confirmed as an applicable treatment for MCDK. The technique was easy to perform, safe and efficient, even when performed by trainees in pediatric urology.

Prospective cohort analyzing risk factors for chronic kidney disease progression in children.

OBJECTIVE: To identify risk factors for chronic kidney disease progression in Brazilian children and to evaluate the interactions between factors. METHODS: This was a multicenter prospective cohort in São Paulo, involving 209 children with CKD stages 3-4. The study outcome included: (a) death, (b) start of kidney replacement therapy, (c) eGFR decrease >50% during the followup. Thirteen risk factors were tested using univariate regression models, followed by multivariable Cox regression models. The terms of interaction between the variables showing significant association with the outcome were then introduced to the model. RESULTS: After a median follow-up of 2.5 years (IQR=1.4-3.0), the outcome occurred in 44 cases (21%): 22 started dialysis, 12 had >50% eGFR decrease, seven underwent transplantation, and three died. Advanced CKD stage at onset (HR=2.16, CI=1.14-4.09), nephrotic proteinuria (HR=2.89, CI=1.49-5.62), age (HR=1.10, CI=1.01-1.17), systolic blood pressure Z score (HR=1.36, CI=1.08-1.70), and anemia (HR=2.60, CI=1.41-4.77) were associated with the outcome. An interaction between anemia and nephrotic proteinuria at V1 (HR=0.25, CI=0.06-1.00) was detected. CONCLUSIONS: As the first CKD cohort in the southern hemisphere, this study supports the main factors reported in developed countries with regards to CKD progression, affirming the potential role of treatments to slow CKD evolution. The detected interaction suggests that anemia may be more deleterious for CKD progression in patients without proteinuria and should be further studied.

Development of Height and Body Mass Index After Pediatric Kidney Transplantation: Experience of the Nephrology Pediatric Service at HCFMRP- USP, 2005-2014.

Introduction: Chronic kidney disease in children often determines poor nutritional status. Although renal transplantation (RTx) resolves endocrine and metabolic disorders, growth continues to be suboptimal and excessive weight gain may result in obesity. Objectives: Evaluating the development of height and body mass index in renal transplanted children and adolescents and identifying associated factors with final nutritional status. Methods: We reviewed the medical records of 17 patients with regular follow-ups up to 24 months after RTx. Nutritional status was assessed by height-for-age (H/A) and body mass index-for-age (BMI/A). It was considered catch-up growth the increase in z-score H/A ≥ 0.5 standard deviation. Multiple linear regression was used to estimate the influence of factors clinical and demographic variables on anthropometric indicators at 24 months after RTx. Results: Mean age was 9.1 ± 4.1 years old. Twenty-four months after RTx the mean z-score H/A increased from -2.66 ± 1.66 to -1.93 ± 1.08 (p ≤ 0.05), 47.0% of the patients showed catch-up growth and the same proportion showed z-score H/A < -2. Mean z-score BMI/A increased from -0.48 ± 1.03 at RTx to 0.80 ± 0.94 at third month after RTx (p < 0.001) and remained unchanged up to 24 months. The frequency of weight excess increased from 5.9% at RTx to 41.2% at 24 months. Age (r = -0.66; p = 0.006) and z-score H/A (r = -0.72; p = 0.002) at RTx were inversely associated with growth. Conclusion: Twenty-four months after transplant it was verified inadequate growth to recovery from stunting and excessive weight gain. RTx promoted greater growth in the youngest patients and most stunted at RTx.

Introdução: A doença renal crônica em crianças geralmente determina o comprometimento do estado nutricional. Embora o transplante renal (TxR) resolva os distúrbios endócrinos e metabólicos, o crescimento continua a ser inadequado e o ganho de peso excessivo pode resultar em obesidade. Objetivos: Avaliar a evolução da estatura e do índice de massa corporal de crianças e adolescentes transplantados renais e identificar fatores associados com o estado nutricional final. Método: Foram revisados os prontuários de 17 pacientes com seguimento regular até 24 meses após o (TxR). O estado nutricional foi avaliado por estatura para idade (E/I) e índice de massa corporal para idade (IMC/I). Foi considerado catch-up de crescimento o aumento no escore-z E/I ≥ 0,5 desvios-padrão. A análise de regressão linear múltipla foi utilizada para estimar a influência de variáveis clínicas e demográficas na variação dos indicadores antropométricos aos 24 meses após TxR. Resultados: A média de idade foi 9,1 ± 4,1 anos. Após 24 meses de TxR, a média de escore-z E/I aumentou de -2,66 ± 1,66 para -1,93 ± 1,08 (p ≤ 0,05), 47,0% dos pacientes apresentaram catch-up de crescimento e a mesma proporção apresentou escore-z E/I < -2. A média do escore-z IMC/I aumentou de -0,48 ± 1,03 no TxR para 0,80 ± 0,94 no terceiro mês após TxR (p < 0,001) e manteve-se estável até 24 meses. A frequência de excesso de peso aumentou de 5,9% no TxR para 41,2% aos 24 meses. A idade (r = -0,66; p = 0,006) e o escore-z E/I no TxR (r = -0,72; p = 0,002) foram inversamente associados ao crescimento. Conclusão: Após 24 meses de TxR verificou-se crescimento insuficiente para recuperação do déficit estatural e ganho ponderal excessivo. O TxR promoveu maior crescimento nos pacientes mais jovens e com maior déficit estatural no TxR.

Development of Height and Body Mass Index After Pediatric Kidney Transplantation: Experience of the Nephrology Pediatric Service at HCFMRP- USP, 2005-2014 / Evolução de Estatura e Índice de Massa Corporal Após Transplante Renal Pediátrico: Experiência do Serviço de Nefrologia Pediátrica do HCFMRP-USP, 2005-2014

Abstract Introduction: Chronic kidney disease in children often determines poor nutritional status. Although renal transplantation (RTx) resolves endocrine and metabolic disorders, growth continues to be suboptimal and excessive weight gain may result in obesity. Objectives: Evaluating the development of height and body mass index in renal transplanted children and adolescents and identifying associated factors with final nutritional status. Methods: We reviewed the medical records of 17 patients with regular follow-ups up to 24 months after RTx. Nutritional status was assessed by height-for-age (H/A) and body mass index-for-age (BMI/A). It was considered catch-up growth the increase in z-score H/A ≥ 0.5 standard deviation. Multiple linear regression was used to estimate the influence of factors clinical and demographic variables on anthropometric indicators at 24 months after RTx. Results: Mean age was 9.1 ± 4.1 years old. Twenty-four months after RTx the mean z-score H/A increased from -2.66 ± 1.66 to -1.93 ± 1.08 (p ≤ 0.05), 47.0% of the patients showed catch-up growth and the same proportion showed z-score H/A < -2. Mean z-score BMI/A increased from -0.48 ± 1.03 at RTx to 0.80 ± 0.94 at third month after RTx (p < 0.001) and remained unchanged up to 24 months. The frequency of weight excess increased from 5.9% at RTx to 41.2% at 24 months. Age (r = -0.66; p = 0.006) and z-score H/A (r = -0.72; p = 0.002) at RTx were inversely associated with growth. Conclusion: Twenty-four months after transplant it was verified inadequate growth to recovery from stunting and excessive weight gain. RTx promoted greater growth in the youngest patients and most stunted at RTx.

Resumo Introdução: A doença renal crônica em crianças geralmente determina o comprometimento do estado nutricional. Embora o transplante renal (TxR) resolva os distúrbios endócrinos e metabólicos, o crescimento continua a ser inadequado e o ganho de peso excessivo pode resultar em obesidade. Objetivos: Avaliar a evolução da estatura e do índice de massa corporal de crianças e adolescentes transplantados renais e identificar fatores associados com o estado nutricional final. Método: Foram revisados os prontuários de 17 pacientes com seguimento regular até 24 meses após o (TxR). O estado nutricional foi avaliado por estatura para idade (E/I) e índice de massa corporal para idade (IMC/I). Foi considerado catch-up de crescimento o aumento no escore-z E/I ≥ 0,5 desvios-padrão. A análise de regressão linear múltipla foi utilizada para estimar a influência de variáveis clínicas e demográficas na variação dos indicadores antropométricos aos 24 meses após TxR. Resultados: A média de idade foi 9,1 ± 4,1 anos. Após 24 meses de TxR, a média de escore-z E/I aumentou de -2,66 ± 1,66 para -1,93 ± 1,08 (p ≤ 0,05), 47,0% dos pacientes apresentaram catch-up de crescimento e a mesma proporção apresentou escore-z E/I < -2. A média do escore-z IMC/I aumentou de -0,48 ± 1,03 no TxR para 0,80 ± 0,94 no terceiro mês após TxR (p < 0,001) e manteve-se estável até 24 meses. A frequência de excesso de peso aumentou de 5,9% no TxR para 41,2% aos 24 meses. A idade (r = -0,66; p = 0,006) e o escore-z E/I no TxR (r = -0,72; p = 0,002) foram inversamente associados ao crescimento. Conclusão: Após 24 meses de TxR verificou-se crescimento insuficiente para recuperação do déficit estatural e ganho ponderal excessivo. O TxR promoveu maior crescimento nos pacientes mais jovens e com maior déficit estatural no TxR.

Complications after bladder augmentation in children.

PURPOSE: Bladder augmentation is an effective surgical procedure for increasing bladder capacity and reducing pressure on the urinary system. It is indicated for patients with anomalies such as spina bifida, myelomeningocele, urethral valve and bladder exstrophy, who progress with low tolerance of medication. CASES: This was a retrospective study conducted on pediatric patients submitted to bladder augmentation from 2000 to 2011. RESULTS: 34 patients aged 4 to 17 years were submitted to bladder augmentation, 30 of them with an ileal loop and 4 with a ureter.A continent urinary shunt was performed in 16 patients, the Mitrofanoff conduit was associated in 15, and the Macedo technique was used in one. Mean follow-up was 34.35 months (1 to 122 months). Mean creatinine was 1.5 ng/ml (0.4 to 7.5 ng/ml) preoperatively and 1.78 ng/ml postoperatively. Three patients required a renal transplant during follow-up. There was improvement or resolution of vesicoureteral reflux in 83.5% of the kidneys on the right and in 75% on the left. Bladder capacity increased, on average, from 152.5 ml to 410 ml. The main complications were vesical lithiasis in 3 patients and conduit perforation in one. CONCLUSION: Bladder augmentation showed good results in this series, preserving renal function in most of the patients.

Complications after bladder augmentation in children

PURPOSE : Bladder augmentation is an effective surgical procedure for increasing bladder capacity and reducing pressure on the urinary system. It is indicated for patients with anomalies such as spina bifida, myelomeningocele, urethral valve and bladder exstrophy, who progress with low tolerance of medication. CASES : This was a retrospective study conducted on pediatric patients submitted to bladder augmentation from 2000 to 2011. RESULTS : 34 patients aged 4 to 17 years were submitted to bladder augmentation, 30 of them with an ileal loop and 4 with a ureter.A continent urinary shunt was performed in 16 patients, the Mitrofanoff conduit was associated in 15, and the Macedo technique was used in one. Mean follow-up was 34.35 months (1 to 122 months). Mean creatinine was 1.5 ng/ml (0.4 to 7.5 ng/ml) preoperatively and 1.78 ng/ml postoperatively. Three patients required a renal transplant during follow-up. There was improvement or resolution of vesicoureteral reflux in 83.5% of the kidneys on the right and in 75% on the left. Bladder capacity increased, on average, from 152.5 ml to 410 ml. The main complications were vesical lithiasis in 3 patients and conduit perforation in one. CONCLUSION : Bladder augmentation showed good results in this series, preserving renal function in most of the patients.

Postnatal evaluation of intrauterine hydronephrosis due to ureteropelvic junction obstruction.

PURPOSE: Fetal hydronephrosis is a frequent finding due to advances in prenatal ultrasonography. The definition of fetal and neonatal urinary tract obstruction is a very difficult task requiring confirmation of reduced renal function and hydronephrosis. In this study we followed a series of consecutive patients with intrauterine hydronephrosis that persisted during post-natal life. METHODS: 116 newborns with antenatal hydronephrosis diagnosed by ultrasound and submitted to a specific post-natal evaluative protocol with a follow-up period of 6 years. RESULTS: In 45 (38.8%) of 116 patients, ureteropelvic junction (UPJ) obstruction was confirmed and surgical correction of the UPJ obstruction was done in 19 patients. From 26 children who were initially submitted to non-surgical treatment, only 6 (23%) needed a surgical approach during follow up. Overall analysis showed that surgery was performed in 25 patients with UPJ obstruction, and the others 20 patients were kept under clinical observation, since normal renal function was confirmed by scintigraphy scans. CONCLUSION: Fetal hydronephrosis due to UPJ obstruction deserves careful postnatal evaluation. UPJ obstruction is the most frequent anomaly and its surgical treatment has very precise indications. The evaluative protocol was useful in identify patients that could be followed-up with a non-surgical approach.

Postnatal evaluation of intrauterine hydronephrosis due to ureteropelvic junction obstruction / Avaliação pós-natal de hidronefrose intra-uterina por estenose da junção ureteropiélica

PURPOSE: Fetal hydronephrosis is a frequent finding due to advances in prenatal ultrasonography. The definition of fetal and neonatal urinary tract obstruction is a very difficult task requiring confirmation of reduced renal function and hydronephrosis. In this study we followed a series of consecutive patients with intrauterine hydronephrosis that persisted during post-natal life. METHODS: 116 newborns with antenatal hydronephrosis diagnosed by ultrasound and submitted to a specific post-natal evaluative protocol with a follow-up period of 6 years. RESULTS: In 45 (38.8%) of 116 patients, ureteropelvic junction (UPJ) obstruction was confirmed and surgical correction of the UPJ obstruction was done in 19 patients. From 26 children who were initially submitted to non-surgical treatment, only 6 (23%) needed a surgical approach during follow up. Overall analysis showed that surgery was performed in 25 patients with UPJ obstruction, and the others 20 patients were kept under clinical observation, since normal renal function was confirmed by scintigraphy scans. CONCLUSION: Fetal hydronephrosis due to UPJ obstruction deserves careful postnatal evaluation. UPJ obstruction is the most frequent anomaly and its surgical treatment has very precise indications. The evaluative protocol was useful in identify patients that could be followed-up with a non-surgical approach.

OBJETIVO: Com a ampla utilização dos exames ultrassonográficos na avaliação pré-natal, é frequente o diagnóstico da hidronefrose fetal. A definição de obstrução do trato urinário no periodo pós-natal necessita da confirmação de redução da função renal além da hidronefrose. Neste estudo, acompanhamos uma série de pacientes consecutivos com hidronefrose intra-útero que persistiu no periodo pós-natal. MÉTODOS: 116 recém-nascidos com hidronefrose pré-natal diagnosticada pela ultrassonografia foram submetidos a protocolo específico de avaliação e companhados pelo periodo de 06 anos. RESULTADOS: Em 46 (38,8%) dos 116 pacientes foi confirmado o diagnóstico de estenose da junção ureteropiélica (JUP). Conforme os resultados do protocolo aplicado a correção cirúrgica da estenose da JUP foi realizada em 19 pacientes. Das 26 crianças encaminhadas inicialmente para observação clínica, apenas 6 (23%) necessitaram cirurgia durante o seguimento ambulatorial. Na análise geral, o procedimento cirúrgico para correção da estenose da JUP foi indicado em 25 pacientes. Nas outras 20 crianças não houve necessidade da realização da cirurgia. CONCLUSÃO: a hidronefrose fetal requer cuidadosa avaliação pós-natal. A estenose da junção pielo-ureteral é a anomalia mais frequente como causa da hidronefrose, e sua correção cirúrgica tem indicações precisas. O protocolo aplicado foi útil em diferenciar pacientes que não necessitaram cirurgia para tratamento da estenose da JUP.

Etiopathogenesis of hepatic osteodystrophy in Wistar rats with cholestatic liver disease.

The pathophysiology of hepatic osteodystrophy (HO) remains poorly understood. Our aim was to evaluate bone histomorphometry, biomechanical properties, and the role of the growth hormone (GH)/insulin-like growth factor-I (IGF-I) system in the onset of this disorder. Forty-six male Wistar rats were divided into two groups: sham-operated (SO, n = 23) and bile duct-ligated (BDL, n = 23). Rats were killed on day 30 postoperatively. Immunohistochemical expression of IGF-I and GH receptor was determined in liver tissue and in the proximal growth plate cartilage of the left tibia. Histomorphometric analysis was performed in the right tibia, and the right femur was used for biomechanical analysis. The maximal force at fracture and the stiffness of the mid-shaft femur were, respectively, 53% and 24% lower in BDL compared to SO. Histomorphometric measurements showed low cancellous bone volume and decreased cancellous bone connectivity in BDL, compatible with osteoporosis. This group also showed increased mineralization lag time, indicating disturbance in bone mineralization. Serum levels of IGF-I were lower in BDL (basal 1,816 +/- 336 vs. 30 days 1,062 +/- 191 ng/ml, P < 0.0001). BDL also showed higher IGF-I expression in the liver tissue but lower IGF-I and GH receptor expression in growth plate cartilage than SO. Osteoporosis is the most important feature of HO; BDL rats show striking signs of reduced bone volume and decreased bone strength, as early as after 1 month of cholestasis. The endocrine and autocrine-paracrine IGF-I systems are deeply affected by cholestasis. Further studies will be necessary to establish their role in the pathogenesis of HO.

Glucocorticoid receptors, in vitro steroid sensitivity, and cytokine secretion in idiopathic nephrotic syndrome.

BACKGROUND: Glucocorticoids (GC) represent the mainstay of treatment of idiopathic nephrotic syndrome (INS) and might be involved in the pathogenesis of the disease. We evaluated basal secretion of cortisol, number and affinity of glucocorticoid receptors, dexamethasone (Dex)-mediated inhibition of concanavalin-A (Con-A)-stimulated peripheral blood mononuclear cell (PBMC) proliferation, and cytokine secretion in patients with INS. METHODS: Blood and saliva were obtained from 20 INS patients in relapse and 11 control patients. Cortisol concentrations were measured by radioimmunoassay. PBMC were isolated for binding and in vitro GC sensitivity assays. Cytokines were measured in supernatants of PBMC culture by enzyme-linked immunosorbent assay (ELISA). RESULTS: Salivary cortisol concentrations were similar in INS patients and control patients. Density and affinity of GC receptors were similar in steroid-sensitive (SS) patients and control, whereas in steroid-resistant (SR) patients they were variable. Lymphocyte proliferation after Con-A stimulation was inhibited by Dex in a dose-dependent manner in control and SS patients. Control and all clinically SS patients were steroid-sensitive by in vitro test, but control patients significantly presented more suppression of PBMC proliferation compared with SS patients. Basal- and Con-A-stimulated interleukin (IL)-6, IL-10, interferon (IFN)-gamma, and tumor necrosis factor (TNF)-alpha levels were similar in control and INS patients, and all cytokines but IL-10 were significantly inhibited by Dex 10-6 mol/L. In SR patients, cytokine secretion remained elevated after treatment with high doses of Dex. CONCLUSION: Abnormalities of number and affinity of the GC receptor and altered secretion of cytokines may be involved in tissue sensitivity to GC in INS patients.

Vesicostomia cutânea em crianças / Cutaneous vesicostomy in children

Objetivo: Avaliar os resulados obtidos com a vesicostomia cutânea como derivaçäo urinária temporária em crianças. Casuística e métodos: Avaliaçäo de 20 crianças (17 meninos e 3 meninas) portadoras de anomalias congênitas do trato urinário e submetidas à vesicostomia cutânea por quadros repetidos de infecçäo urinária sintomática e/ou reduçäo da funçäo renal, associados a acidose metabólica e acentuada uretero-hidronefrose. Resultados: A anomalia mais freqüente foi válvula de uretra posterior (12 pacientes), seguida por refluxo vésico-ureteral em 7 e válvula de uretra anterior em 1. Desses pacientes, 16 apresentavam uréia e creatinina séricas elevadas no momento do diagnóstico. Após a derivaçäo observou-se reduçäo da ectasia do sistema urinário em todos os pacientes...

O desenvolvimento renal: aspectos anatômicos e funcionais / Renal development: anatomic and functional aspects

Este artigo de revisäo enfoca o processo de maturaçäo renal do ponto de vista anatômico e funcional, durante o período intrauterino e pós-natal. O rim do recém-nascido caracteriza-se por baixa taxa de filtraçäo glomerular e um balanço positivo de sódio, potássio e fósforo, necessário para o crescimento. Apresenta também baixa capacidade de concentraçäo urinária, que o torna mais suceptível à desidrataçäo; menor habilidade em manter a homeostase ácido-base e tendência à acidose; perdas urinárias maiores de glicose, aminoácidos e ácido úrico, que diminuem com a maturaçäo. O recém-nascido pré-termo apresenta características ainda mais peculiares devido a maior imaturidade renal. O conhecimento das particularidades da funçäo renal do recém-nascido a termo e prematuro é importante para o adequado manejo nutricional e tratamento farmacológico

Refluxo vesico-uretral primario: resultado do tratamento de 40 criancas / Primary vesico-uretral reflux: result of the treatment of forty children

Sao avaliados os resultados obtidos em 40 criancas (16 meninos e 24 meninas tratadas de refluxo vesico-uretral primario. Em todos os pacientes a avaliacao radiologica do trato urinario foi realizada para investigacao de causa de infeccao urinaria. O tratamento inicial foi cirurgico em 19 e conservador em 21 pacientes. Destes, 11 (52,4%) apresentaram regressao completa da patologia dois anos apos o diagnostico; quatro (19%0 estao aguardando exames controles e seis (28,6%) foram posteriormente operados. Nos pacientes cirurgicos foirealizada plastica anti-refluxo em 31 ureteres, com uma recidiva (96,7% de cura). E dada enfase a avaliacao precoce do trato urinario em criancas com infeccao urinario e ao emprego do tratamento conservador no refluxo vesico-uretral de baixa intensidade